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Background: Prophylactic colectomy or proctocolectomy is standard treatment for colorectal manifestation of familial adenomatous polyposis (FAP), a dominantly inherited disorder for which the risk of developing colorectal cancer in an untreated patient is close to 100 percent. Hereditary nonpolyposis colorectal cancer (HNPCC) is also dominantly inherited but has a lower risk of colorectal cancer than FAP and does not have a clinically obvious phenotype. The role of prophylactic colectomy in patients with HNPCC is controversial., Purpose: This study was performed to examine the outcome of colectomy and ileorectal anastomosis (IRA) so its use as a prophylactic procedure can be better evaluated., Methods: Records of all patients undergoing IRA for FAP between 1985 and 1993 were reviewed. Demographic data and data about the operation were collected. Surgical outcome data included length of hospital stay, complications, bowel function, quality of life, and patient satisfaction., Results: There were 51 patients with a median age of 28 years; 24 were male. All but eight patients were asymptomatic, and all had less than 1,000 polyps in the resected specimen. Mean surgery time was 3.5 hours, mean blood loss was 406 ml, and median length of hospital stay was seven days. There were no deaths, and eight patients (16 percent) had complications. Mean number of stools per day after median follow-up of 4.2 years was 3.6. Only 11 patients had nighttime stooling. Four patients reported seepage, 9 had some incontinence, and 16 had urgency. Quality of life, rated on a scale of 0 to 10, was 7 or above in 44 of 48 assessed patients. Quality of health was rated 7 or higher in all 48 patients, energy level was 7 or higher in 39 patients, and overall happiness with surgery was 7 or higher in 47 patients., Conclusions: Colectomy and IRA is a relatively safe operation that results in minimum disturbance of bowel function. Patient satisfaction is usually high. Prophylactic colectomy can be offered to HNPCC gene carriers with a greater understanding of the likely outcome of surgery.

Purpose: A case of a patient with familial adenomatous polyposis (FAP) is reported, in whom adenomas developed in an ileal pelvic pouch six years after it was made. This case is reported to serve as a warning that restorative proctocolectomy, a relatively recent addition to surgical options for FAP, does not remove the risk of metachronous intestinal neoplasia; it merely defers it., Methods: Case of a patient with pouch polyposis was reviewed, and patient was prospectively studied after three months of sulindac therapy., Results: Polyps not removed at first examination became much less prominent. Literature review reveals only one study of adenomas in pelvic pouches, with 7 cases of 38., Conclusion: Proctocolectomy and ileal pouch-anal anastomosis does not cure FAP, and multiple polyps can occur in the ileal pouch.

Purpose: The aim of our study was to evaluate the safety and functional outcome of restorative proctocolectomy (RP) without diversion., Methods: Fifty patients underwent RP without diversion for ulcerative colitis (82 percent), familial adenomatous polyposis (12 percent), and indeterminate colitis (6 percent). The perioperative course and functional outcome of these patients were compared with another group of 50 patients undergoing RP with diverting ileostomy during the same time period (1989-1991) and closely matched for age, gender, surgeon, diagnosis, extent and duration (median, 10 years) of colitis, prior colectomy (approximately 22 percent), steroid use (40 percent), type of pouch, distance of ileal pouch-anal anastomosis from the dentate line (median, 1.5 cm), and the duration of follow-up (median, 12 months). All patients had a stapled ileal pouch-anal anastomosis without mucosectomy and a smooth conduct of the operation., Results: There was no operative mortality. Anastomotic leaks and pelvic abscess were more common in patients without ileostomy (7/50 or 14 percent vs. 2/50 or 4 percent); 8 of these 9 patients were taking > or = 20 mg of prednisone/day. Septic complications requiring relaparotomy (6 percent vs. 0 percent), prolonged ileus, and fever of unknown origin (10 percent vs. 4 percent) were also more common in patients without ileostomy. Despite similar functional results at 6 weeks and at 12 months after initial pouch function, patients without ileostomy had a poorer quality of life index (5 vs. 8; 10 being best) in the early period (0-6 weeks) of pouch function., Conclusion: In equally favorable cases, RP without diversion is not as safe as RP with diversion, especially in patients taking > or = 20 mg of prednisone/day.

Unlabelled: This article examines the effect of ileal pouch-anal (n = 134) and coloanal (n = 16) anastomoses on resting anal canal pressures in 150 patients., Methods: Patients underwent anal manometry before ileal pouch-anal anastomosis (IPAA) and coloanal anastomosis (CAA) and again six weeks after ileostomy closure following these procedures. A water-perfused catheter system with four radial ports was used for manometry, pressures being recorded during both station and continuous pull through., Results: Patients with IPAA were younger than those with CAA (34 years vs. 50 years) and had a different ratio of hand-to-stapled anastomosis (1:2.6 vs. 1.3:1). All CAA patients had had rectal cancer while IPAA patients suffered mainly from ulcerative colitis (n = 114) or familial polyposis (n = 10). The mean preoperative resting pressure for all patients was 79 mmHg (75-87, 95 percent confidence limit) and the mean fall in this pressure after surgery was 25 mmHg (-21 to -29, 95 percent confidence limit). There was no difference in preoperative pressure or fall between handsewn and stapled anastomoses, or between IPAA and CAA., Conclusion: There was a significant relationship between preoperative pressure and change in pressure that held true for all subgroups (change = -0.7 x preoperative pressure + 31, r = 0.69). Analysis of the functional results confirmed that patients with high preoperative pressure are at risk for severe falls after surgery and are not guaranteed a good result. Conversely, patients with low preoperative pressures may actually have an increase with surgery and are not always incontinent. Patients with low preoperative anal resting pressures should not be denied anastomosis to the anus if they are continent.

Purpose: The aim of this study was to compare treatment outcomes in the management of pelvic abscess (PA) after rectal surgery., Methods: Over a 12-year period all PAs occurring in the patients undergoing colorectal resection were retrospectively reviewed. The APACHE II Score was used to stratify illness., Results: Postoperative PA developed in 56 patients after cancer (32 percent), ulcerative colitis (26 percent), diverticular disease (24 percent), and Crohn's colitis (18 percent)/surgery. Overall, 24 (43 percent) of PAs were after operations for inflammatory bowel disease and 43 (77 percent) of PAs were after intrapelvic intestinal anastomoses. PAs were treated by 1) antibiotics alone (11/56), 2) percutaneous computerized tomography-guided catheter drainage (13/56), 3) transperineal drainage (15/56), or 4) laparotomy (17/56). Recurrent PAs developed in 11/56 (19 percent) after initial treatment, of which 7 required additional surgery. These recurrences were evenly distributed between treatment groups. There were three deaths as a result of PA, two after laparotomy and one after percutaneous drainage. Long-term sequela in patients with intestinal anastomosis included loss of intestinal continuity (10/43) and anastomotic stenosis (7/43). There was no difference in APACHE II Score among the four treatment groups. The mortality rate was 75 percent among patients whose APACHE II Scores were greater than 15. The development of a PA after colon and rectal surgery was associated with a 5 percent mortality and 41 percent functional morbidity (23 percent permanent stoma and 18 percent symptomatic stricture rate)., Conclusion: Using clinical judgment, if PA is amenable to computerized tomography-guided percutaneous or transperineal drainage, one of these techniques should be attempted initially in the hemodynamically stable nonseptic patient. Long-term functional disability is common after PA in rectosigmoid surgery in patients who undergo pelvic/intestinal anastomosis.

Levator syndrome is a symptom complex of severe pain and pressure in the anorectal area. Electrogalvanic stimulation (EGS) has been proposed as a treatment for this condition. Several reports have described EGS as up to 90 percent "effective" in treating levator syndrome, but the length of follow-up was uncertain or short-term in these studies. The purpose of this study was to examine the long-term benefits of EGS in levator syndrome patients treated at one institution. All patients undergoing EGS for levator syndrome between 1985 and 1991 were studied. Initial complaints, physical examination, number of treatments, procedure tolerance, and long-term benefit were determined through personal interviews and chart reviews. There were 52 patients (63 percent females and 37 percent males) with a median age of 54 years (range, 24-84 years). All patients presented with anorectal pain. Tenderness was localized by examination to the left in 43 percent, to the right in 23 percent, and bilateral in 8.6 percent and was not localized in 2.6 percent. Fifty percent received fewer than four one-hour treatments, 33 percent received four to six treatments, and 17 percent received more than six treatments. Seventy-seven percent felt that the treatment was painless. Follow-up results were as follow: number, 52; percent follow-up, 88; mean follow-up, 28 months (range, 0-71 months); symptoms relieved, 19 percent; partial relief, 24 percent; no relief, 57 percent. Of four patients with a wrong diagnosis, three were ultimately diagnosed with recurrent pelvic cancer and one had an anal fissure. At our institution, EGS was a tolerable treatment, but a substantial number of patients received no benefit. An organic etiology of anorectal pain must always be excluded.

The operation of choice for complete rectal prolapse is controversial. We reviewed 169 patients undergoing 185 surgical procedures for rectal prolapse over a 27-year period. The most common surgical procedure employed was the Ripstein procedure (n = 142) and is the focus of this report. Other surgical procedures used included resection rectopexy (n = 18), anterior resection (n = 7), Altemeier's (n = 9), Delorme's (n = 2), and anal encirclement (n = 7). The median age was 59 years (range, 12-94 years), and the female-to-male ratio was 5:1. The incidence of fecal incontinence, solitary rectal ulcer syndrome, and prior surgery elsewhere for rectal prolapse was 40 percent, 12 percent, and 19 percent, respectively. Operative mortality was 0.6 percent; morbidity was 16 percent. Median follow-up was 4.2 years (range, 1-15 years). Complete recurrence of prolapse after the Ripstein procedure was 8 percent; one-third of these patients recurred 3 to 14 years after surgery. Fecal incontinence improved after the Ripstein procedure or resection rectopexy in about half the patients. Persistence of prior constipation was more common after the Ripstein procedure than after resection rectopexy (57 percent vs. 17 percent; P = 0.03, chi-squared). Fifteen patients developed constipation for the first time after the Ripstein procedure. About one in three patients, irrespective of surgical procedures, remained dissatisfied with the final outcome despite anatomic correction of the prolapse. The Ripstein procedure has proven to be a safe procedure with good anatomic repair of the prolapse and may improve continence. In the presence of constipation, procedures other than the Ripstein procedure may be preferable.

Because Crohn's disease of the small bowel is often diffuse, strictureplasty has been advocated as an alternative or adjunct to resection(s) of strictured segments. We reviewed 116 patients with obstructive Crohn's disease undergoing 452 primary strictureplasties (Heineke-Mikulicz, 405; Finney, 47). The median age was 34 years (range, 13-72 years); the male-to-female ratio 1.4:1; and the median follow-up was three years (range, six months to seven years). Seventy-six patients (66 percent) had at least one previous small bowel resection. Perforative disease was present in 18 patients (15 percent), and synchronous resections were performed in 71 patients (61 percent). The median number of strictureplasties was three (range, 1-15). There was no mortality. Septic complications (intra-abdominal abscess/fistula) occurred in seven patients (6 percent), and reoperation for sepsis was needed in two patients. Relief of obstructive symptoms was achieved in 99 percent of the patients. After surgery, the median weight gain was 4 kg, and two-thirds of the patients were weaned off steroids. Symptomatic recurrence occurred in 28 patients (24 percent), and 17 patients (15 percent) needed reoperation. Rates of restricture and new stricture/perforative disease were 2.8 percent and 24 percent, respectively.

Solitary rectal ulcer syndrome (SRUS) often goes unrecognized or is misdiagnosed. Of 98 patients with a final clinicopathologic diagnosis of SRUS, an initially incorrect diagnosis had been made in 25 patients (26 percent). In these 25 patients with a misdiagnosis, the median age was 43 years and the female-to-male ratio was 3.2:1. The median duration of incorrect diagnosis was five years (range, three months to 30 years), and seven patients received prednisone (> 30 mg/day) for a mistaken diagnosis of inflammatory bowel disease. The main clinical symptoms were rectal bleeding (84 percent) and a disturbance of bowel function (56 percent). Rectal prolapse was present in 13 patients. Original rectal biopsy specimens from 23 patients were reviewed; inadequate specimens and failure to recognize diagnostic features of SRUS contributed to delayed diagnosis in 13 and 10 patients, respectively. The most common clinicopathologic misdiagnoses in SRUS patients with rectal ulcers or mucosal hyperemia were Crohn's disease and mucosal ulcerative colitis. In patients with "polypoid" SRUS, diagnostic confusion was usually with a neoplastic polyp. Persistent bowel symptoms and rectal lesions led to review of the presentations and repeat biopsy directed toward the edge of the rectal ulcers or from within the polypoid or hyperemic rectal lesions, finally establishing the diagnosis of SRUS. Intractable symptoms led to surgery in 15 patients (60 percent), with symptomatic improvement in over two-thirds.

A review of the endoscopy reports and pathology results from esophagogastroduodenoscopy (EGD) of all patients with familial adenomatous polyposis (FAP) undergoing such an examination was performed. Two hundred forty-seven patients were identified, with an overall prevalence of duodenal adenomas of 66 percent and of fundic gland polyps of 61 percent. Analysis of our more recent experience (1986 to 1990) shows the prevalence to be 88 percent and 84 percent, respectively. A normal-appearing papilla was adenomatous in 50 percent of cases. No case of periampullary carcinoma developed in patients under surveillance. Routine EGD is indicated for patients with FAP. Duodenal adenomas and fundic gland polyps will occur in the majority of patients.

One hundred thirty-one patients underwent ileorectal anastomosis (IRA) for Crohn's colitis. Preoperatively, 84 patients (63 percent) were found to have mild or moderate proctitis and 47 (37 percent) had rectal sparing. Sixty-eight (52 percent) had associated small bowel disease, and 20 (15 percent) had perianal disease. Sixty-five IRAs were performed at the time of subtotal colectomy, while 56 were done after previous surgery. Anastomotic leaks occurred in four patients. There were no operative deaths. Thirteen patients (10 percent) with protecting stomas never underwent closure. Among the remaining 118 patients with functioning IRAs, 30 (23 percent) required later proctectomy and 16 (13 percent) required proximal diversion, with the mean period with a functioning IRA in these 46 patients being 4.1 years (range, 6.2 months-12.7 years). An additional 13 patients required preanastomotic resection and neo-IRA, and 11 required proximal small bowel resection. The mean duration of function of all 118 IRAs was 9.2 years. At the time of review, after a mean follow-up of 9.5 years, 72 patients (61 percent) retained a functioning IRA, with 44 being free of disease, while 28 were being treated with steroids or antidiarrheal medication. The mean stool frequency was 4.7 per day. In patients with Crohn's colitis, IRA should be considered as an alternative to proctocolectomy if the rectum is not severely diseased and sphincter function is not compromised.

The aims of this study were to determine whether endoluminal ultrasound (ELUS) could identify various layers of the normal anal canal and to evaluate whether a 10-MHz probe provided better image resolution than a 7-MHz probe. Sonographic anatomy of the anal canal on ELUS was directly correlated with anatomic dissection of various layers (mucosa-submucosa, internal anal sphincter, and external anal sphincter) in cadavers. Sonographic appearance of the anal sphincters was further evaluated in patients by "tagging" various layers using sonodense needles. A higher frequency 10-MHz ultrasound probe (focal length, 1-4 cm) provides improved sonographic images of the anal canal, compared with the 7-MHz probe (focal length, 2-5 cm). ELUS can also successfully identify various structures of the pelvic floor including the puborectalis, urethral sphincter, vagina, and outlines of the pelvis and ischiorectal fossae. Its role in the evaluation of anorectal disorders appears promising.

A retrospective study of 80 patients with biopsy-proven solitary rectal ulcer (SRU) was conducted to review its clinical spectrum. The median follow-up was 25 months. The female-to-male ratio was 1.4:1.0, and the mean age was 48.7 years (range, 14-76 years). Principal symptoms were bowel disturbances (74 percent) and rectal bleeding (56 percent). Twenty-one patients (26 percent) were asymptomatic and required no treatment. A previous "wrong" diagnosis was made in 25 percent. Rectal prolapse was identified in 28 percent (full-thickness, 15 percent; mucosal, 13 percent). The macroscopic appearance of the lesion seen in SRU varied widely and included polypoid lesions in 44 percent (the predominant finding in the asymptomatic group), ulcerated lesions in 29 percent (always symptomatic), and edematous, nonulcerated, hyperemic mucosa in 27 percent. Anorectal manometry provided little helpful information in the patients in whom it was performed. Management by bulk laxatives and bowel retraining led to symptomatic improvement in 19 percent of cases. In 29 percent of cases, symptoms persisted despite endoscopic healing of the lesion. Intractability of symptoms led to surgery in only 27 (34 percent) patients. Depending on the presence or absence of rectal prolapse, rectopexy or a conservative local procedure (such as local excision), respectively, appeared to be the optimal surgical treatment. The polypoid variety tended to respond to therapy more favorably than non-polypoid varieties. Thus, the macroscopic appearance of SRU has a significant bearing on the clinical course, and most cases do not require surgery.

Conventional hemostatic measures are often unsatisfactory in presacral venous bleeding occurring during surgical mobilization of the rectum. We designed a new type of hemorrhage occluder pin, with a ridged shaft, which may be rapidly placed into the sacrum to control hemorrhage. The aims of this study were 1) to assess the best pin shaft length by measuring the thickness of human sacral vertebral bodies, 2) to measure the forces needed to pull the newly designed pin out of the human sacrum compared with conventionally shaped titanium thumbtacks, and 3) to assess clinically the efficacy of the new device. Four fresh cadaveric pelves were isolated and cut on a sagittal plane, and the thickness of each vertebral body was measured. Titanium pins, both with ridged and with smooth shafts, were used. Twelve-millimeter-shaft pins were used for S1 and S2, and 7-mm pins were used for S3, S4, and S5. Pins were inserted into each sacral vertebra, and the forces needed to extract them from the bone were measured by computerized dynamometry. Significantly more force was required to extract ridged vs. smooth pins, both with 12-mm and with 7-mm shafts. There was no significant difference between the forces needed to pull out 12-mm vs. 7-mm pins. The new pin was successfully used to stop presacral hemorrhage in three patients with no complications one, three, and six months after surgery. This newly designed hemorrhage occluder pin may represent an improved method of controlling presacral venous hemorrhage.

Forty of 416 patients with familial adenomatous polyposis were noted to have intra-abdominal desmoid tumors, and a subgroup of 16 were treated with noncytotoxic drug therapy. Drugs used were sulindac (14 patients), sulindac plus tamoxifen (3 patients), indomethacin (4 patients), tamoxifen (4 patients), progesterone (DEPO-PROVERA; Upjohn Co., Kalamazoo, MI) (2 patients), and testolactone (1 patient). Therapy with these drugs for continuous periods of six months or more resulted in three complete and seven partial remissions. When treated patients were compared with untreated patients (n = 12), there were significant benefits for the treated group, both in reduction of desmoid size and in improvement of symptoms, despite the inherent selection bias against this. Sulindac was the only drug used in enough patients to permit independent evaluation of its effect, with one complete and seven partial reductions of tumor size. Some patients had a delayed response to sulindac, with tumor shrinkage occurring after an initial period of tumor enlargement. When using sulindac for the treatment of desmoid tumors, this phenomenon should be considered.

From 1961 to 1989, 67 patients underwent various surgical procedures for psoas abscess. Retrospective analysis was undertaken in an effort to determine optimal surgical therapy. Forty patients were cured with one operation. Twenty-one patients required two operations, four patients required three operations, and two patients required more than three operations. The reason for failure of treatment was failure to resect the diseased bowel or to drain the psoas abscess adequately. A technique to recognize and treat the abscess definitively will be illustrated. The most common etiologies were Crohn's disease in 49 patients, postoperative sepsis in eight patients, and complications of renal disease in four patients. The length of hospital stay ranged from 5 to 392 days (mean, 26 days). There were two deaths. Failure to recognize and treat psoas abscess results in considerable morbidity.

In a retrospective review of 311 patients having subtotal colectomy for ulcerative colitis, information on the fate of the rectal stump was obtained in 288. Proctectomy was performed in 159 patients (55 percent); for persistent proctitis in 118 (41 percent), cancer prophylaxis in 37 (13 percent), and cancer in four (1.4 percent). One hundred twenty-two patients (42 percent) had ileorectal anastomoses. Eighty-four of these (69 percent) retained a functioning ileorectal anastomosis at the time of follow-up or death, one to 22 years later, and an additional six patients (5 percent) had a satisfactory ileorectal anastomosis for five to 14 years before proctectomy. Cancer developed in the rectal stump in nine patients (3.1 percent), underscoring the need for either proctectomy (total or mucosal) or long-term surveillance of the retained rectum. However, subtotal colectomy, by permitting ileorectal anastomosis or other sphincter-preserving surgery at a later date, does have a definite place in many patients requiring surgery for ulcerative colitis.

A retrospective review was conducted of 326 patients undergoing intersphincteric rectal excision for ulcerative colitis. Seventy-five patients (Group A) had rectal excision with closure of the pelvic peritoneum and packing of the pelvic space via an open perineal wound. One hundred sixty-nine patients (Group B) had excision without pelvic peritoneal closure, but with the levators and subcutaneous tissue closed and with transabdominal sump suction drainage of the pelvic space. Complete healing for Groups A and B occurred by three months in 42 and 79 percent, respectively, and by six months in 56 and 89 percent. Thirty-one percent of Group A and 9 percent of Group B were unhealed at one year, and/or required further surgery. When all 326 patients were considered, healing was achieved at three, six, and 12 months, for packed and for closed wounds, in 42 percent and 79 percent, 55 percent and 89 percent, 66 percent and 91 percent, respectively. All these differences are highly significant (P less than 0.0001). The incidence of small-bowel obstruction requiring surgery during follow-up was similar whether the pelvic peritoneum was closed (15.5 percent) or left open (15.7 percent). Other factors which adversely affected perineal wound healing were younger age, a short history of disease, a two-stage proctocolectomy especially for persistent severe rectal disease, and the presence of perianal disease.

To evaluate the outcome of surgery for ulcerative colitis in pediatric and adolescent patients, the experience at the Cleveland Clinic Foundation was reviewed retrospectively. Fifty-nine percent of the patients presented with acute toxic colitis and sepsis; 94 percent underwent staged procedures with creation of a temporary or permanent ileostomy. Sepsis was the most frequent postoperative complication (38 percent), and accounted for all three deaths (5 percent). Long-term disability was minimal, and 90 percent of the patients were fully active at the time of follow-up.